BACKGROUND:Extracorporeal photochemotherapy (photopheresis), an immune-modulating therapy, has been demonstrated to elicit a therapeutic response in the treatment of several autoimmune disorders. We evaluated the use of photopheresis in the treatment of patients with progressive systemic sclerosis (PSS; scleroderma).

METHODS:Five patients with early-onset, diffuse PSS were treated with photopheresis on 2 successive days monthly for an average of 59 months (range 54-89 months). We initially reported the response this group of patients had to photopheresis treatment at an average of 11 months (range 6-21 months).

RESULTS:An improvement or stabilization was noted in most patients in skin thickening, joint mobility, pulmonary function studies, oral aperture, functional index, as well as symptoms including Raynaud's phenomenon, dyspnea, fatigue, dysphagia, arthralgias, and cutaneous ulcers. Renal function tests remained within normal range. A total of 296 monthly treatments were administered without significant toxicity.

CONCLUSIONS:This study suggests that extended use of extracorporeal photochemotherapy in the management of early-onset, diffuse PSS is well tolerated and may provide an increasingly beneficial clinical outcome.

译文

背景:体外光化学疗法(光透疗法)是一种免疫调节疗法,已被证明在多种自身免疫性疾病的治疗中引起治疗反应。我们评估了光采疗法在进行性系统性硬化症(PSS;硬皮病)患者中的应用。

方法:对5例早发,弥漫性PSS患者进行了光采疗法。每月连续2天,平均59个月(范围54-89个月)。我们最初报告了该组患者平均11个月(6到21个月)对光穿刺治疗的反应。

结果:大多数患者均出现了改善或稳定皮肤增厚,关节活动度,肺功能研究,口腔孔径,功能指数以及包括雷诺现象,呼吸困难,疲劳,吞咽困难,关节痛和皮肤溃疡在内的症状的患者。肾功能检查保持在正常范围内。总共进行了296个月的每月治疗,无明显毒性。

结论:这项研究表明,在早期发作,弥漫性PSS的管理中长期使用体外光化学疗法具有良好的耐受性,并且可能会提供越来越有益的临床结果。

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